ObjectiveTo determine the ability of cardiopulmonary exercise testing (CPET) to distinguish chronic thromboembolic pulmonary hypertension (CTEPH) from chronic thromboembolic disease (CTED). MethodsFifty patients diagnosed with CTED and fifty-eight patients with CTEPH in the the First Affiliated Hospital of Guangzhou Medical University from April 2019 to February 2022 were retrospectively included. The basic characteristics including age, gender, body mass index, symptom duration, and N-terminal prohormone of brain natriuretic peptide (NTpro-BNP), parameters of arterial blood gas analysis, right heart catheterization, echocardiography, pulmonary function, and CPET were all compared between patients with CTED and those with CTEPH. ResultsPatients with CTEPH displayed longer symptom duration, increased NTpro-BNP, decreased arterial partial pressure of oxygen, larger right atrial and ventricular diameters, and impaired worse resting pulmonary diffusing function compared with those with CTED (P<0.05). However, there was no statistically significant difference in the resting pulmonary ventilation function between the two groups (P>0.05). Among the CPET parameters of the CTEPH group, peak exercise oxygen uptake per kilogram, oxygen uptake at anaerobic threshold, oxygen pulse, oxygen uptake efficiency slope and oxygen saturation were all decreased, while the minute ventilation-carbon dioxide production at anaerobic threshold (VE/VCO2@AT) and VE/VCO2 slope were increased (P<0.05). However, there was no statistically significant difference in heart rate reserve and breathing reserve (P>0.05). Furthermore, VE/VCO2@AT showed high accuracy for distinguishing CTEPH from CTED (sensitivity, 0.825; specificity, 0.860; and the area under the receiver operating characteristic curve 0.897). ConclusionsPatients with CTEPH showed more significant decreased exercise endurance, diffusion dysfunction, and hypoxemia during exercise and insufficient increase in cardiac output compared with CTED patients. Therefore, it is feasible to apply CPET as a new objective examination to distinguish CTED from CTEPH.
Abstract: Objective To analyze the results of preoperative evaluation of the operability of chronic thromboembolic pulmonary hypertension (CTEPH), and to find parameters to define the inconsistency between the operable lesions of CTEPH and pulmonary vascular resistance (PVR). Methods A total of 133 patients with CTEPH admitted into Anzhen Hospital were enrolled for preoperative assessment of operability from March 2002 to May 2010. There were 86 males and 47 females with an age of 49.10±22.70 years. The patients were divided into operable group (group A, n=82，age of 47.80±21.60 years) and inoperable group (group B, n=51, age of 50.30±23.40 years) according to the assessment suggestion. We evaluated the occluded pulmonary segment（OPS） of all the patients through pulmonary ventilation/perfusion scintigraphy， and measured the plasma aminoterminal Btype natriuretic peptide (NT-pro BNP) and PVR. Then the ratio of NT-pro BNP to OPS and PVR to OPS were calculated. [CM(159mm]Results Out of the 82 patients with CTEPH in group A, 81 were positive in the anesthesia test and were subject to pulmonary thromboendarterectomy(PTE). In the whole cohorts of operated patients, there was one early death due to persistent pulmonary hypertension and right heart failure. The specificity of the anesthesia test was 98.78%. Eighty patients (98.77%) were followed up in this group for a period of 1 to 95 months (42.70±28.40 months). During the followup, there was one late death due to pulmonary artery hypertension crisis. Among the 51 patients with CTEPH in group B, there were 32 patients (62.75%) with surgically inaccessible lesions, 13 patients (25.49%) with surgical accessible CTEPH concomitant with severe diseases, and 6 patients (11.76%) with inconsistency between the surgical accessible lesion and high PVR. The ratio of NT-pro BNP to OPS and PVR to OPS for the 81 positive patients in group A was in the range of 80-150 pg·ml-1/OPS and 50-100 dyn·s·cm-5/OPS, respectively. The ratio of NT-pro BNP to OPS (315.00±83.00 pg·ml-1/OPS vs. 115.60±40.50 pg·ml-1/OPS, P=0.000) and PVR to OPS (190.00±57.00 dyn·s·cm－5/OPS vs. 76.40±26.30 dyn·s·cm-5/OPS, P=0.000) for the 6 patients with incosistency between the surgical accessible lesion and high PVR in group B were significantly higher than that for the 81 positive patients in group A. Conclusion Surgically inaccessible CTEPH lesions, CTEPH concomitant with severe diseases, and inconsistency between surgical accessible lesion and high PVR are the three most frequent reasons for denying PTE procedure. The ratio of NTpro BNP to OPS and PVR to OPS may serve as the parameters to define the inconsistency between the surgical accessible lesion and high PVR. Anesthesia test before the PTE procedure may serve as the last evaluation method for the assessment of the operability of CTEPH.
Objective To investigate the effect of low-flow inhaling NO for short time on postoperative cardiac and pulmonary functions in infants with congenital ventricular septal defect complicated with severe pulmonary hypertension. Methods Forty-five patients with congenital ventricular septal defect complicated with severe pulmonary hypertension from May 2014 to May 2016 in our hospital were enrolled. There were 19 males and 26 females, whose age ranged from 1 to 22 months (average age: 7.2±14.4 months) and weight ranged from 2.7 to 10.5 kg (average weight: 6.8±3.6 kg). The patients were randomly divided into three groups (n=15 in each): the blank group, the prior inhalation group and the posterior inhalation group. The blank group did not inhale NO, and the prior inhalation group inhalated NO for 10 min after tracheal and intubation. After the opening of the aorta, the posterior inhalation group inhaled NO for 10 min. The concentration of NO was 20 × 10–6. The pressure ratio of pulmonary circulation/systematic circulation, heart index and oxygenation index were calculated and the troponin value of the three groups was monitored 10 min after returning to intensive care unit (ICU) and postoperatively 1 h, 3 h and 24 h. Differences among above indicators between three groups were compared. Results The troponin value of the posterior inhalation group within 3 h increased most, followed by the blank group and the prior inhalation group. Postoperatively 1 h and 3 h, the troponin value of the prior inhalation group was significantly less than that of the blank group and posterior inhalation group (P<0.01) and the value on postoperative 24 h in each group was lower than that on postoperative 3 h. The cardiac index of prior inhalation group was higher than that of the blank group and the posterior inhalation group at each time point. Postoperatively 3 h and 24 has well as 10 min after returning to ICU, the cardiac index in prior inhalation group was significantly higher than that of the posterior inhalation group (P<0.05). The pressure ratio of pulmonary circulation/systematic circulation of posterior inhalation group increased more than that of blank group; the differences in two groups were significant between postoperative 3 h and 10 min after returning to ICU (P<0.01). There was no statistical significance in the pressure ratio on postoperative 24 h and 10 min after returning to ICU (P>0.05) in three groups. The index of oxygenation of the prior inhalation group was higher than that of the blank group and the posterior inhalation group and statistically different from that of posterior inhalation group (P<0.05). Conclusion Inhaling NO 10 min preoperatively can reduce the injury to the heart and lung function effectively, but the result is the opposite when inhalating NO 10 min after aorta opening.
Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close monitoring of cardiac function and regular scan of fetal assessment. The prenatal and postpartum integrated diagnosis and treatment model and the development of intrauterine treatment technology reduce the incidence of congenital heart disease in adults from the source through fetal intervention. Other complications such as arrhythmia, infective endocarditis, cerebrovascular accidents, and other medical underlying metabolic diseases also challenge future diagnosis and treatment. The incidence and epidemiology of adult congenital heart disease, pulmonary hypertension and end-stage heart failure complications, as well as prenatal and postpartum integrated diagnosis and treatment and intrauterine treatment are summarized in this review.
Objective To investigate the effects of simvastatin on the collagen synthesis of rat pulmonary arterial smooth muscle cells ( PASMCs ) induced by hypoxia. Methods Under hypoxic condition, rat PASMCs were cultured with different concentrations of simvastatin. Collagen synthesis of PASMCs with or without simvastatin were measured by 3H-proline incorporation assay. The mRNA expression of TGF-β1 and the contents of super oxide dismrtase ( SOD) ,malondialdehyde ( MDA) in mediumwere also measured. Results The incorporation data of 3H-TdR in the hypoxia group was significantly increased as compared with that in the control group ( P lt;0. 01) , and simvastatin significantly reduced the incorporation data of 3H-TdR induced by hypoxia. The expression of TGF-β1 mRNA in the hypoxia group was significantly increased as compared with that in the control group ( P lt; 0. 01 ) , and simvastatin could significantly inhibited hypoxia-induced expression of TGF-β1 mRNA in a dose-dependent manner. Compared with the hypoxia group, the expression of TGF-β1 mRNA decreased by 55% in simvastatin( 10 - 6mol /L) group ( P lt; 0. 01) , and by 70% ( P lt; 0. 01) in simvastatin ( 10 - 5mol /L) group. Compared with the control group, the activity of SOD was reduced and the contents of MDA were increased significantly in the hypoxia group. Simvastatin can increase the activity of SOD and reduced the content of MDA in a dose-dependent manner. Conclusions Simvastatin can decreases collagen synthesis of PASMCs. This effect might be explained that simvastatin can reduce lipid peroxide and expression of TGF-β1 mRNA.
Objective To investigate the incidence and management of CTEPH in the Department of Pulmonary and Critical Care Medicine in Xijing Hospital to enrich the epidemiological data of chronic thromboembolic pulmonary hypertension (CTEPH) in China.Methods We conducted a retrospective study to investigate the incidence and management of CTEPH in the Department of Pulmonary and Critical Care Medicine in Xijing Hospital from 2008 to 2012. Results The incidence of CTEPH was 5.24% . About 62.90% of venous thromboembolism/pulmonary embolism (VTE/PE) patients were unprovoked, and about 53.85% of CTEPH patients was unprovoked. About 38% of CTEPH patients had no history of VTE, and 62% of CTEPH patients had no history of acute pulmonary embolism. None of the CTEPH patients was treated by pulmonary thromboendarterctom (PTE) , and about 53.85% of patients were only given anticoagulant monotherapy. Conclusions The incidence of CTEPH is higher in our hospital than reported. This phenomenon may be related to the lack of awareness of risk factors of CTEPH and the insufficient thrombolytic and anticoagulant therapy to acute pulmonary embolism. It’s very urgent to standardize the diagnosis and management of CTEPH in pulmonologists.
Objective To make an individualized treatment plan concerning a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension. Methods Based on the clinical questions raised by a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension, we searched The Cochrane Library (Issue 3, 2009), MEDLINE (1980 to June 2009), ACP Journal Club (1991 to June 2009), and Chinese Journal Fulltext Database (1994 to June 2009) for systematic reviews, randomized controlled trials (RCTs) and case-control studies. The quality of the included studies was assessed. Results A total of 9 RCTs, 1 health economic evaluation, 1 meta analysis, and 2 systematic reviews were considered eligible. The evidence indicated that the use of ECMO in infants of PPHN had shown a decreased risk of death, but not cost-saving from a societal perspective; iNO treatment could improve the PaO2 and resulted in a reduction in the incidence of requirement for ECMO; there were not randomized controlled trials regarding the treatment of PPHN by hyperventilation, high-frequency ventilation, alkali infusion, pulmonary vasodilators (magnesium sulfate, tolazoline, prostaglandin or prostacyclin, milrinone), surfactant therapy; oral sildenafil could lower oxygenation index (OI) and result in a reduction in the incidence of death. The individualized treatment plans of oral sildenafil were developed based on the available evidence, existing conditions of the hospital, and the values of children with families. After 1 month of treatment, the FiO2 returned to normal and symptoms were alleviated. Conclusion The treatment efficacies and the survival rates in meconium aspiration syndrome combined with PPHN have been improved by determining an individualized treatment plan according to evidence-based methods.
Medical nitric oxide (NO) flow control system plays an important role in lowering pulmonary hypertension. The design requirements, overall scheme, delivery system and hardware circuits of a medical NO flow control system were introduced in this paper. Particularly, we proposed the design of NO delivery system and hardware circuits in detail. To deliver nitric oxide of a variable concentration, the designed system needs to work with a ventilator. The system can adjust and monitor the inhaled nitric oxide concentrations and send out sound and light alarms when the inhaled nitric oxide concentrations are out of the set range. To validate reliability and efficacy, we measured specifications such as linearity, stability and response time of the proposed NO flow control system by continuously administering nitric oxide into inspiratory circuit to deliver nitric oxide of variable concentrations to a test lung. The experiments showed that these specifications can meet the desired requirements.
ObjectiveTo study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension.MethodsThe clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg.ResultsWindows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal.ConclusionAPW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.
Pulmonary hypertension due to left heart disease (PH-LHD) is the most common in various types of pulmonary hypertension. Although there are many treatments for pulmonary hypertension, it may be harmful when we adopt treatment without detrimental diagnosis and classification of pulmonary hypertension. Therefore, it is very crucial to have accurate diagnosis and classification of pulmonary hypertension before making treatment decisions. However, there are still some difficulties in the classification of pulmonary hypertension in clinical work. It is a great challenge with limited treatment to solve the PH-LHD which often has complicated pathophysiological mechanisms of precapillary and postcapillary pulmonary hypertension. Here, we review the research status of PH-LHD.